Total branched-chain amino acids requirement in patients with maple syrup urine disease by use of indicator amino acid oxidation with L-[1-13C]phenylalanine.
D000293:Adolescent; D000328:Adult; D000597:Amino Acids, Branched-Chain / Q000008:administration & dosage* / Q000378:metabolism*; D000601:Amino Acids, Essential / Q000378:metabolism; D004734:Energy Metabolism; D005260:Female; D006801:Humans; D008297:Male; D008375:Maple Syrup Urine Disease / Q000175:diagnosis* / Q000378:metabolism*; D008657:Metabolic Clearance Rate; D009751:Nutritional Requirements; D010084:Oxidation-Reduction; D010649:Phenylalanine; D011865:Radioisotope Dilution Technique
D000597:Amino Acids, Branched-Chain; D000601:Amino Acids, Essential; D010649:Phenylalanine